-Both children and adults are more vulnerable to infections and have a harder time fighting them off. This is the result of damage to the spleen from the sickled red cells, which prevents the spleen from destroying bacteria in the blood. -Infants and young children, especially, are susceptible to bacteria (infections that can kill them in as little as 9 hours from onset of fever).

In 1995, an NHLBI clinical trial of hydroxyurea in adults over the age of 18 demonstrated, for the first time, an effective therapy for reducing painful episodes in severely affected adults with sickle cell anemia. Although the FDA has approved hydroxyurea for adults with sickle cell anemia, there is concern about the potential toxicity of hydroxyurea in children, and the effects on growth and development. It is not recommended for children at this time, however, clinicians are interested in looking for benefits of preventing organ damage and improving long-term survival especially in children. Further clinical research is needed in children to investigate the effectiveness of hydroxyurea. This will be of great benefit if spleen function can be preserved, and the deleterious effects to the central nervous system, lungs and kidneys can be prevented (Cure Research for Sickle Cell Disease). Several other drugs under investigation (erythropoietin, butyrate and related compounds) may work together with hydroxyurea to make it more effective. Bone marrow transplantation can really cure the disease, but the risks are high, and only a small number of young patients are good candidates for that form of experimental therapy. Some day gene therapy may provide a safe and effective cure, but that is some years in the future (Liu, Agarwal, Kmiec, and Davis).

The ultimate cure for sickle cell anemia may be gene therapy. In sickle cell anemia, the gene that switches on production of adult hemoglobin shortly before birth is defective. Two approaches to gene therapy are being explored. Some scientists are looking into whether correcting this gene and inserting it into the bone marrow of people with sickle cell anemia will result in the production of normal adult hemoglobin. Others are looking at the possibility of turning off the defective gene and simultaneously reactivating another gene that turns on production of fetal hemoglobin. In both cases, the research is at a very early stage (Cure Research for Sickle Cell Anemia). Although the genetic defect that causes sickling was identified more than 40 years ago, until very recently, research into the development of treatments for the disease was hampered by the lack of an animal model that could be used to test experimental drugs and gene therapy. Recently, however, scientists were able to genetically engineer a line of mice that exhibit some of the characteristics of sickle cell anemia in much the same way humans do. Progress is being made, however, and there is a real possibility of an eventual clinical cure for sickle cell anemia.

-If one parent has sickle cell anemia (HbSS) and the other is completely unaffected (HbAA) then all the children will have sickle cell trait. None will have sickle cell anemia. The parent who has sickle cell anemia (HbSS) can only pass the sickle hemoglobin gene to each of their children.

Some topics in this essay:
Cell Society, Cell Anemia, Chest Syndrome, FDA Consumer, Campbell Reece, Cleve Lemerond, Davis Regular, Parker Parker, Kmiec Davis, sickle cell, Segments DNA, cell anemia, sickle cell anemia, blood cells, fetal hemoglobin, sickle cell trait, cell trait, people sickle cell, people sickle, red blood, red blood cells, chance child, gene therapy, hemoglobin gene, parent sickle cell,